Neurology· Topic 10.3 Headache and facial pain· 10.3.3

Cluster headache

Cluster headache is a trigeminal autonomic cephalalgia causing severe unilateral orbital or temporal pain lasting 15 to 180 minutes, up to eight times daily, with ipsilateral autonomic features. First-line acute treatment is 100% oxygen inhalation or subcutaneous sumatriptan; verapamil is first-line prevention.

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What this article covers

  • Overview

    Definition: cluster headache is the most common trigeminal autonomic cephalalgia (TAC), a primary headache syndrome producing severe, strictly unilateral orbital, supraorbital, or temporal pain…

  • Epidemiology

    Sex distribution: cluster headache is markedly more common in men than women, unlike migraine which favors women.

  • Risk factors

    Male sex: the strongest demographic risk factor.

  • Pathophysiology

    Trigeminal-autonomic reflex activation: pain arises from activation of the trigeminal nerve's ophthalmic division, which through the trigeminal-autonomic reflex arc activates the cranial…

  • Clinical features

    Diagnostic criteria (ICHD-3): at least five attacks fulfilling: severe or very severe unilateral orbital, supraorbital, and/or temporal pain lasting 15 to 180 minutes untreated; accompanied by at…

  • Differential diagnosis

    Migraine: episodes last hours to days rather than minutes, pain is often throbbing and can be bilateral, and patients seek quiet and darkness rather than pacing; autonomic features can overlap but are usually milder.

  • Diagnosis

    Clinical diagnosis: cluster headache is diagnosed clinically using the ICHD-3 criteria above; no laboratory or imaging test confirms the diagnosis.

  • Management

    First-line abortive therapy, given at attack onset, single dose per attack: 100% oxygen by non-rebreather mask, high flow, for 15-20 minutes.

  • Complications

    Suicidality: the severity of pain during cluster attacks has led to the historical label "suicide headache," and clinicians should screen for mood symptoms and suicidal ideation, particularly during active bouts.

  • Prognosis

    Episodic form: most patients have episodic cluster headache, with bouts lasting weeks to months followed by prolonged remission; bout frequency and duration are often but not always stable over a patient's lifetime.

  • Prevention

    Bout-based prophylaxis: preventive therapy (verapamil, titrated toward 180-480 mg PO daily, with or without a 2-week glucocorticoid bridge) should begin as soon as a new bout starts and continue…

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